Episode 2: Sensory Neurons

Many cells in the body seem to function  quite well despite low levels of frataxin.   However certain cells of the spinal cord,  brain, and heart are badly affected. Some of these cells stop functioning over time and  some needed frataxin to develop correctly in the   first place. This seems to be the case for some  of the very specialized nerve cells in the spinal   cord: the primary sensory neurons. These special  cells collect information about what's going on   inside and outside the body and they bring that  information to a specific place in the spinal   cord called the dorsal root ganglia or drg. The  drgs transfer this information to the brain.   This transfer of the information allows  us to sense where our body is in space.   This is referred to as proprioception. Dr. Massimo  Pandolfo and his colleagues at Université Libre de Bruxelles in Belgium received a grant from FARA  to use induced pluripotent stem cells, or iPSCs,   to grow and study fa sensor neurons.  iPSCs have the potential to mature   into almost any type of cell in the body;  including heart brain or spinal cord cells   and in another video we learned how we can use  the iPSCs to study fa. Dr. Pandolfo and his team   are trying to figure out the changes that  occur in fa sensory neurons during development   and after the cells mature as they address  the question of why these particular cells   are so affected by frataxin loss. This information  will help us better understand how we might stop   or reverse the damage caused by FA. Many thanks  to all the patients and families who donated   cells and to the community for raising funds and  awareness to support this important research tool. The project we just learned about is one seed,  one tool, funded by your donations, championed   by your engagement, and your direct participation  in FA research that is necessary to plant, tend,   and nourish our garden of therapeutics. Thank you  FA community for bringing us closer to treatments.

Channel: Friedreich's Ataxia Research Alliance

Views: 12,853

Keywords: friedreich's ataxia, fa, curefa, rare diseases, movement disorder, ataxia

Id: B2fSsOLoH2M

Length: 3min 27sec (207 seconds)

Published: Wed Jan 27 2021